Beham A, Badve S, Suster S, Fletcher C D
Institute of Pathology, University of Graz Medical School, Austria.
Histopathology. 1993 Apr;22(4):335-41. doi: 10.1111/j.1365-2559.1993.tb00132.x.
Solitary myofibroma is a recently described, benign neoplasm of superficial soft tissue, which represents the adult counterpart of infantile myofibromatosis and is poorly recognized. Eleven new cases are presented herein. The patients were mostly adults with ages ranging from 13-64 years. They presented with a solitary, usually painless nodule of variable duration in the skin or oral cavity. Histologically, each lesion had a biphasic pattern with spindle cells forming fascicular or whorled areas and rounded, more primitive cells arranged around small vessels, forming haemangiopericytoma-like areas. The characteristic zonation of infantile myofibromatosis was often less marked in adult lesions and there was a haphazard arrangement of the fascicular and pericytic areas in some cases. Hyalinization, especially of the peripheral spindle-celled areas, was frequent. Immunohistochemically, the spindle cells were desmin negative but muscle actin (HHF-35 and IA4) positive. The rounded cells were both desmin and actin negative. Electronmicroscopy confirmed the myofibroblastic/fibroblastic nature of tumour cells in two cases. Although vascular invasion was seen in two cases, solitary adult-type myofibroma pursues a benign clinical course.
孤立性肌纤维瘤是一种最近才被描述的浅表软组织良性肿瘤，它是婴儿肌纤维瘤病的成人对应物，目前对其认识不足。本文报告11例新病例。患者多为成年人，年龄在13至64岁之间。他们表现为皮肤或口腔内单个、通常无痛的结节，病程不一。组织学上，每个病变都有双相模式，梭形细胞形成束状或漩涡状区域，圆形、更原始的细胞围绕小血管排列，形成类似血管外皮细胞瘤的区域。婴儿肌纤维瘤病的特征性分区在成人病变中往往不那么明显，在某些情况下，束状和周细胞区域排列杂乱。透明变性，尤其是外周梭形细胞区域的透明变性很常见。免疫组化显示，梭形细胞结蛋白阴性，但肌动蛋白（HHF-35和IA4）阳性。圆形细胞结蛋白和肌动蛋白均为阴性。电镜检查在两例中证实了肿瘤细胞的肌成纤维细胞/成纤维细胞性质。虽然两例中可见血管侵犯，但孤立性成人型肌纤维瘤临床病程呈良性。
